Telias Michael, Nawy Scott, Kramer Richard H
Department of Molecular and Cell Biology, University of California, Berkeley, CA, United States.
Helen Wills Neuroscience Institute, University of California, Berkeley, CA, United States.
Front Neurosci. 2020 Dec 18;14:618019. doi: 10.3389/fnins.2020.618019. eCollection 2020.
Vision impairment and blindness in humans are most frequently caused by the degeneration and loss of photoreceptor cells in the outer retina, as is the case for age-related macular degeneration, retinitis pigmentosa, retinal detachment and many other diseases. While inner retinal neurons survive degeneration, they undergo fundamental pathophysiological changes, collectively known as "remodeling." Inner retinal remodeling downstream to photoreceptor death occurs across mammalian retinas from mice to humans, independently of the cause of degeneration. It results in pervasive spontaneous hyperactivity and membrane hyperpermeability in retinal ganglion cells, which funnel all retinal signals to the brain. Remodeling reduces light detection in vision-impaired patients and precludes meaningful vision restoration in blind individuals. In this review, we summarize current hypotheses proposed to explain remodeling and their potential medical significance highlighting the important role played by retinoic acid and its receptor.
人类的视力障碍和失明最常见的原因是视网膜外层光感受器细胞的退化和丧失，年龄相关性黄斑变性、色素性视网膜炎、视网膜脱离及许多其他疾病皆是如此。虽然视网膜内层神经元在退化过程中存活下来，但它们会发生根本性的病理生理变化，统称为“重塑”。从小鼠到人类的所有哺乳动物视网膜中，光感受器死亡后都会发生视网膜内层重塑，且与退化原因无关。它会导致视网膜神经节细胞普遍出现自发性活动亢进和膜通透性增加，从而将所有视网膜信号传入大脑。重塑会降低视力受损患者的光检测能力，并使盲人无法实现有意义的视力恢复。在本综述中，我们总结了目前提出的用以解释重塑现象的假说及其潜在医学意义，强调了视黄酸及其受体所起的重要作用。
